Juvenile gangrenous vasculitis of the scrotum: A rare cause of genital ulcer

Juvenile gangrenous vasculitis of the scrotum is a rare entity of unknown aetiology which should be considered in the differential diagnosis of genital ulcers, mainly with those caused by sexually transmitted infections. The authors report a case of a 23-year-old patient who presented a single painful scrotal ulcer developing shortly after an episode of pharyngotonsillitis. The ulcer was treated with oral corticosteroids with total healing and no relapse in over two years of follow-up.     

Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis – part I

The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.     

Antineutrophil cytoplasmic antibody-associated vasculitis with alveolar hemorrhage and ruptured renal aneurysm: A case report and literature review

Rationale:Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is characterized by necrotizing damage to small-vessel vasculitis and mainly occurs in the kidney or lung. We report a rare case of AAV manifesting as alveolar hemorrhage and a renal aneurysm.Patient concerns:A 50-year-old Chinese man presented with repeated coughing, expectoration, fever, hypoxemia, and respiratory failure. The patient suffered from rupture of the renal aneurysm during immunosuppressive therapy.Diagnosis:Considering the clinical picture (fever, progressive hypoxemia, renal insufficiency, hemorrhagic bronchoalveolar lavage fluid, and left retroperitoneal hematoma) along with cANCA-PR3 positivity, and lung biopsy findings, the patient was finally diagnosed with granulomatosis with polyangiitis complicated by alveolar hemorrhage and renal aneurysm.Interventions:The patient was initially treated with immunosuppressive therapy combined with plasma exchange and subsequently with renal arterial embolization due to rupture of the renal aneurysm.Outcomes:The general condition and inflammatory reaction improved with immunosuppressive therapy combined with plasma exchange. Unfortunately, the patient did not respond to treatment and eventually died of respiratory failure and acute kidney injury after the rupture of the renal aneurysm.Lessons:We encountered unprecedented difficulties and challenges with renal aneurysm rupture. The possibility of aneurysmal rupture should be carefully considered and frequently checked for immunosuppressive therapy for AAV.     

An autopsy case of Balamuthia mandrillaris amoebic encephalitis,a rare emerging infectious disease,with a brief review of the cases reported in Japan

Balamuthia mandrillaris is an amoeba found in fresh water and soil that causes granulomatous amoebic encephalitis. We report herein an autopsy case of B. mandrillaris amoebic encephalitis, which was definitely diagnosed by PCR. An 81‐year‐old man, who had Sjögren's syndrome, manifested drowsiness 2 months before his death with progressive deterioration. Neuroimaging demonstrated foci of T2‐ and fluid‐attenuated inversion recovery high and T1 low‐intensity with irregular post‐contrast ring enhancement in the cerebral hemisphere, thalamus and midbrain. Pathologically, multiple hemorrhagic and necrotic lesions were found in the cerebrum, thalamus, midbrain, pons, medulla and cerebellum, which were characterized by liquefactive necrosis, marked edema, hemorrhage and necrotizing vasculitis associated with the perivascular accumulation of amoebic trophozoites, a few cysts, and the infiltration of numerous neutrophils and microglia/macrophages. The trophozoites were ovoid or round, 10–60 μm in diameter, and they showed foamy cytoplasm and a round nucleus with small karyosome in the center. The PCR and immunohistochemistry from paraffin‐embedded brain specimens revealed angioinvasive encephalitis due to B. mandrillaris. Human cases of B. mandrillaris brain infection are rare in Japan, with only a few brief reports in the literature.     

Cyclosporin A in the treatment of cutaneous vasculitis. Clinical and cellular effects

Background Treatment of cutaneous vasculitis(CV) is an unsettled issue. Cyclosporin A (Cy-A) has been successfully used in dermatoses with immunologic background. some systemic vasculitides and Behcet's disease. Objective To check the therapeutic potential of Cy-A in severe or recalcitrant cases of CV was considered of interest. The effects on the in situ cell populations involved in the inflammatory-immune events were further investigated. Methods An open clinical trial (12 cases) was carried out. An immunohistochemical study was also performed, evaluating skin specimens from established lesions, before and 48 h after Cy-A first administration. The effect on endothelial adhesion molecules, integrin bearing leukocytes, T-cells, lymphomononuclear cells and dendrocytes in the dermis as well as on epidermal antiyenic determinants focused our interest. Results Reduced endothelial ICAM-1 expression was shown. Cy-A was proved to adversely affect b2 integrin expression and therefore adhesion pathways. T-cells, epidermal dendritic cells and dendrocytes were found decreased. On clinical grounds, a very early therapeutic response was noted. Tolerance was good in the dose of 5mg/kg/day. Relapses, however, have not been avoided. Conclusion Cy-A seems to be very effective on both the clinical and cellular level where inflammatory-immunologic events seem to be temporarily inhibited.     

Atypical presentation of adenosine deaminase 2 deficiency with bi‐allelic ADA2 mutation

Herein, we report a case of VAIHS with atypical clinical presentation of perianal abscess, fistula fever, and bi‐cytopenia including pathogenic ADA2 mutation suggesting that ADA2 deficiency be considered as a differential diagnosis of enlarging cutaneous abscess with no evidence of wound healing in the setting of leukopenia and neutropenia.     

大血管血管炎发病机制中的遗传因素

大血管血管炎包括大动脉炎和巨细胞动脉炎,病因涉及遗传、感染、环境、免疫反应等因素,发病机制仍待明确.在遗传背景研究方面发现,大动脉炎与HLA-Ⅰ类基因区(HLA-B?52)关系最为密切,参与炎症反应的相关基因(IL12B、IL6、RPS9/LILRB3)也发挥着重要作用.在巨细胞动脉炎中,HLA-Ⅱ类(HLA-DRB1...